RESULTS Atrophy of the caudate nuclei was identified in all Huntington patients. The frontal horn distance/intercaudate distance (1.64 +/- 0.39) and bicaudate (0.205 +/- 0.060) ratios of the patients with juvenile Huntington disease were found to be significantly different from those of healthy children and that of those patients with Leigh/Wilson disease. The 3 patients with Huntington disease who underwent MR evaluation were noted to have increased proton density- and T2-weighted signal in the caudate nuclei and putamina.

CONCLUSION As in adult patients, the use of frontal horn distance/intercaudate distance and bicaudate ratios are helpful for the diagnosis of Huntington disease in pediatric patients. On MR, increased proton density- and T2-weighted signal in the atrophic caudate nuclei and putamina are additional features of juvenile Huntington disease.